case report anterior segment findings in vitamin a...

Case ReportAnterior Segment Findings in Vitamin A Deficiency:A Case Series

Pierangela Rubino, Paolo Mora, Nicola Ungaro, Stefano A. Gandolfi, and Jelka G. Orsoni

Ophthalmology Unit, Department of General and Specialized Surgery, University Hospital of Parma,Via Gramsci 14, 43126 Parma, Italy

Correspondence should be addressed to Pierangela Rubino; [email protected]

Received 23 March 2015; Revised 16 June 2015; Accepted 15 September 2015

Academic Editor: Shigeki Machida

Copyright © 2015 Pierangela Rubino et al. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.

Vitamin A deficiency is a rare but vision threatening disorder in the developed world, which can lead to blindness for severekeratomalacia with cornea scarring and perforation or night blindness due to impaired dark adaptation. Conversely, the disease isquite common in developing countries, as a consequence of chronic malnutrition. The correct diagnosis and therapy with promptvitaminA supplementation avoid blindness.We report a series of 3 local cases with different age and causes for vitaminA deficiency.The diagnostic workup, therapy, and prognosis are discussed.

1. Introduction

Vitamin A deficiency is a common cause of childhood blind-ness in the world. Over 124 million children worldwideare esteemed to have vitamin A deficiency for chronicmalnutrition, and ocular manifestations occur in 5 millionpeople annually.Thedisorder is rare in developedworld [1, 2].

In developed countries nutrition deviancies are reportedin a variety of pathologies such as Celiac disease, biliaryobstruction, cystic fibrosis, chronic liver diseases includingalcoholism, inflammatory bowel disease with malabsorption,or following pancreatic or intestinal surgery. Vitamin Adeficiency secondary to fatty acids malabsorption in bowelbypass surgery has also been reported in the literature,although incidence is poorly known [3].

Vitamin A deficiency leads to a variety of ocular manifes-tations including cornea and conjunctival xerosis, keratiniza-tion of the conjunctiva, keratomalacia and potentially cornealperforation, retinopathy, visual loss, and nyctalopia.

We present 3 cases of Italian patients with vitamin Adeficiency who responded to vitaminA oral supplementationand had good recovery of visual function.

2. Materials and Methods

Case 1. A 4-year-old Italian child was referred to our servicefor foreign body sensation, dry eye, and ocular redness of

two-week duration. His diet was poor in fruits and vegetablesand he suffered from multiple allergic diseases and foodintolerances (i.e., eggs and lactose).

His best corrected visual acuity (BCVA)was 20/20 in botheyes with normal motility and fundus. Slit-lamp examinationshowed the keratinization of the temporal and nasal con-junctiva with bilateral Bitot’s spots; the cornea was normal,Figures 1(a) and 1(b).

The serum vitamin dosage was 0.12 𝜇g/mL; normal value(NV) is 0.20–0.80.

Therapywith oral vitaminA (RetinolAcetate) 150.000 IU/mL/day for 3 days, then with 50.000 IU/mL/week for twoweeks, and finally with multivitamin supplementation andcorrect diet for two months was given, in association withtopical treatment with vitamin A ointment (retinoic acid0.1%) twice a day and intensive preservative-free lubricants.

Rapid improvement and progressive resolution of con-junctiva keratinization were documented after 2 weeks andat two months, respectively, Figures 2(a) and 2(b).

The level of vitamin A improved to 0.39 𝜇g/mL (N.V.0.20–0.80).

Case 2. A 47-year-old Italian woman was referred to us forred eye, dry eye, and foreign body sensation in both eyesand persistent pain in right eye. The patient had a priordiagnosis of type I Arnold Chiari Malformation (she wasaffected by syringomyelia) and she underwent bowel bypass

Hindawi Publishing CorporationCase Reports in Ophthalmological MedicineVolume 2015, Article ID 181267, 6 pageshttp://dx.doi.org/10.1155/2015/181267

2 Case Reports in Ophthalmological Medicine

(a) (b)

Figure 1

(a) (b)

Figure 2

surgery 6 years before. BCVAwas 20/32 in right eye and 20/20in left eye; slit-lamp examination showed wrinkling of theconjunctiva and keratinization in both eyes and sterile andperipheral ulcer in right eye (Figures 3 and 4). Fundus in botheyes and cornea in left eye were normal.

Laboratory tests revealed severe vitamin A deficiencywith blood level to 0.1 𝜇g/mL.

Treatment with vitamin A ointment and preservative-free eye drops was immediately started and, after laboratoryevidence, oral supplementation was added as in the casedescribed above. The conjunctiva and the cornea improvedand, after 7-8 weeks, the cornea ulcer in right eye andthe conjunctiva keratinization disappeared and a completeresolution of clinical manifestations was observed (Figures5 and 6). Serum level of vitamin A recovered to 0.35 𝜇g/mLafter 4 weeks.

Blood levels of vitamin were periodically verified (8–12 weeks) and, eventually, new therapeutic regimen wasrepeated.

Case 3. A 79-year-old Italian woman was referred us forirritation and excessive tearing in both eyes. Symptoms hadrecently been exacerbated but the concomitant primary openangle glaucoma with long-lasting therapy with beta-blockersand carbonic anhydrase inhibitor eye drops (dorzolamide 2%and carteolol) misled the attention of several ophthalmolo-gists.

At slit-lamp examination the patient presented kera-tinization of the conjunctiva and shortening of the lower con-junctival fornix in both eyes, partial corneal keratinization inthe left eye (Figures 7(a), 7(b), and 8), and glaucomatous opticneuropathy in both eyes. BCVAwas 20/63 in the right eye and20/100 in the left eye.

The patient was hospitalized for anorexia, weight loss of10 kg in the last six months, diarrhea, and anaemia.

Diagnosis of Crohn disease was made after exclusion ofliver or pancreatic cancer and any other form of tumourincluding lymphatic tumour, Celiac disease as malabsorptioncause; she started treatment with oral steroids and Salazopy-rin.

Vitamin A dosage was 0.20𝜇g/mL after hemotransfusionfor severe anaemia.

She was treated with vitamin A (Retinol Acetate) oral200.000 IU/mL/day for 3 consecutive days and then with50.000 IU/mL/week for two weeks and then with IV multi-vitamin complex (containing 83.000UI vitamin A, 16.600UIvitamin D, and 16mg of B and E complex).

Treatment with vitaminA ointment and preservative-freeeye drops was started too.

The keratinization of the cornea and conjunctivaimproved in both eyes (Figures 9, 10, and 11), but systemicclinical condition of this critical patient worsened after 3months and she died. The dosage of vitamin A after 1 month

Case Reports in Ophthalmological Medicine 3

(a) (b)

(c)

Figure 3: Right eye before therapy.

(a) (b)

(c)

Figure 4: Left eye before therapy.


(a)

(b)

Figure 5: Right eye after therapy.

Figure 6: Left eye after therapy.

of therapy was 0.41. BCVA was 20/63 in right eye and 20/80in left eye.

3. Results and Discussion

Vitamin A is a fat-soluble vitamin introduced with foodsfrom animal sources, such as meat, liver, eggs, fish, and milkas retinol form, and from vegetable sources, yellow fruits asprovitamin carotene form. The retinol is transformed in theliver in retinoic acid, the active form, that induces the celldifferentiation and modulates gene expression. Vitamin A isnecessary for vision, epithelial tissue differentiation, skeletaltissue maintenance, spermatogenesis, placenta generation,and maintenance.

(a)

(b)

Figure 7: Right eye before therapy.

Figure 8: Left eye before therapy.

The ocular symptoms and sign of vitaminAdeficiency arevariable, potentially affecting all the epithelial cells of the eye.Disorders may range from simple dryness of the conjunctivaand the cornea up to xerosis, severe keratomalacia, cornealscarring, and perforation; visual functionmay also be affectedwith night blindness for impaired dark adaptation and retinalphotoreceptors pigment epithelial cell damage.

In our cases the diagnosis of vitamin A deficiency wassuspected on clinical signs and symptoms in patients withhistory of malabsorption, in cases 2 and 3, and insufficientintake in case 1; the serum vitamin A dosage was performedto confirm clinical suspect. Dark-adapted electroretinogramscan also aid the diagnosis if nyctalopia is present, and we didnot perform it because our patients did not show nyctalopia.

Case Reports in Ophthalmological Medicine 5

(a)

(b)

Figure 9: Right eye after therapy.

The World Health Organization advised that treatmentis a single oral dose of 200.000 IU vitamin A, followed bya further dose the following day and a final dose severalweeks later [4]. We treated our patients with oral vitamin A(Retinol Acetate) 200.000 IU/mL/day for 3 consecutive daysand then with 50.000 IU/mL/week for two weeks in the adultpatients and 150.000 IU/mL/day in the child patient. Theserum vitamin A dosage was repeated in the follow-up.

Topical treatment with vitamin A ointment and pre-servative-free eye drops was performed in each patient.In cases 1 and 2 a complete healing of the cornea andconjunctival lesions was achieved, while in case 3 only apartial restoration of the corneal and conjunctival xerosiswas observed before patient’s death for compromised generalcondition.

The goal of this report is to focus the ophthalmologistsattention on a rare problem that can otherwise be facedin the common activity. They should be able to recogniseearly symptoms and signs of keratomalacia and conjunctivakeratinisation and to think about vitamin A deficiencyin particular in patients affected by Celiac disease, biliaryobstruction, cystic fibrosis, chronic liver disease, alcoholism,and inflammatory bowel disease or patients who underwentpancreatic or intestinal surgery. Nowadays, digesting bypasssurgery is a common option for obesity treatment and it isknown to be associated with hypovitaminosis A. Dermato-logical and ophthalmological symptoms may develop alsomany years after surgery [5–9]; vitamin A deficiency shouldbe always considered in such patients.

Figure 10: Left eye after 3 weeks of therapy.

Figure 11: Left eye after 8 weeks of therapy.

4. Conclusion

Although uncommon, vitamin A deficiency should be con-sidered among the possible differential diagnoses for oph-thalmic disturbances in patients with malnutrition or malab-sorption conditions, or in patient with bowel bypass surgery.Specific questions should be included in theirmedical historyevaluation. Adequate supplementation of vitamin A in thesepatients may valuably resolve the clinical and functionalalterations when the damage is at an early stage.

Conflict of Interests

The authors declare that there is no conflict of interestsregarding the publication of this paper.

References

[1] J. H. Humphrey, K. P. West Jr., and A. Sommer, “VitaminA deficiency and attributable mortality among under-5-year-olds,” Bulletin of the World Health Organization, vol. 70, no. 2,pp. 225–232, 1992.

[2] J. Smith and T. L. Steinmann, “Vitamin A deficiency and theeye,” International Ophthalmology Clinics, vol. 40, no. 4, pp. 83–91, 2000.

[3] K. C. Zalesin, W. M. Miller, B. Franklin et al., “Vitamin Adeficiency after gastric bypass surgery: an underreported post-operative complication,” Journal of Obesity, vol. 2011, Article ID760695, 4 pages, 2011.


[4] D. R. Paranjpe, D. C. Newton, and A. E. A. Pyott, “Nutritionaldisorders,” in Cornea, J. H. Krakmer, M. J. Mannis, and E. J.Holland, Eds., pp. 721–732, Mosby Elsevier, 3rd edition, 2011.

[5] G. H. Slater, C. J. Ren, N. Siegel et al., “Serum fat-solublevitamin deficiency and abnormal calcium metabolism aftermalabsorptive bariatric surgery,” Journal of GastrointestinalSurgery, vol. 8, no. 1, pp. 48–55, 2004.

[6] Y. Spits, J.-J. De Laey, and B. P. Leroy, “Rapid recovery ofnight blindness due to obesity surgery after vitamin A repletiontherapy,” British Journal of Ophthalmology, vol. 88, no. 4, pp.583–585, 2004.

[7] R. Enat, A. Nagler, L. Bassan et al., “Night blindness and livercirrhosis as late complications of jejunoileal bypass surgery formorbid obesity,” Israel Journal of Medical Sciences, vol. 20, no. 6,pp. 543–546, 1984.

[8] W. B. Lee, S. M. Hamilton, J. P. Harris, and I. R. Schwab, “Ocularcomplications of hypovitaminosis A after bariatric surgery,”Ophthalmology, vol. 112, no. 6, pp. 1031–1034, 2005.

[9] T. Chae and R. Foroozan, “Vitamin A deficiency in patientswith a remote history of intestinal surgery,” British Journal ofOphthalmology, vol. 90, no. 8, pp. 955–956, 2006.

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